DIVERSE NEUROLOGICAL MANIFESTATION OF HYPERIMMUNOGLOBULIN-E SYNDROME: A CASE SERIES
نویسندگان
چکیده
Hyper-immunoglobulin E (IgE) is a disorder of immune system characterized by assemblage symptoms including recurrent skin and pulmonary infections, skeletal anomalies elevated serum IgE (>2000 IU/ml). More recently, apart from the typical features, neurological manifestations have also been described central nervous facial nerve palsy demyelinating disorders. Here we describe three patients with hyper syndrome having diverse manifestation varying CNS infection to basal ganglia stroke progressive leukoencephalopathy.
منابع مشابه
Hyperimmunoglobulin E syndrome.
Hyperimmunoglobulin E (HIE) syndrome is a primary immunodeficiency disorder characterized by recurrent bacterial infections in presence of very high serum Ig E levels. We are reporting a nine-year-old child with HIE syndrome and reviewing literature on this disease.
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Hyperimmunoglobulin E syndrome is a primary immunodeficiency disease characterized by markedly high titers of serum immunoglobulin E (IgE), chronic eczema, recurrent staphylococcal infections, pneumatoceles, reduced neutrophil chemotaxis, and variable impaired T cell function. There are no clinical tools for diagnosis and definitive laboratory investigation. Variability of presentation makes it...
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The hyper-IgE syndromes are rare, complex primary immunodeficiencies characterized by clinical manifestation diversity, by particular susceptibility to staphylococcal and mycotic infections as well as by a heterogeneous genetic origin. Two distinct entities--the classical hyper-IgE syndrome which is inherited in an autosomal dominant pattern and the autosomal recessive hyper-IgE syndrome--have ...
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Hyperimmunoglobulin E recurrent infection syndrome (HIES) is characterized by recurrent skin and lung infections, eczema, elevated serum immunoglobulin E (IgE) levels (>2000 IU/mL), various connective tissue, skeletal, and vascular abnormalities.(1) We describe herein two brothers with HIES and documented the complications and management of such involvement.
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ژورنال
عنوان ژورنال: Pakistan postgraduate medical institute
سال: 2023
ISSN: ['2710-1924']
DOI: https://doi.org/10.51642/ppmj.v34i02.609